lunes, 17 de septiembre de 2012

Stone People: living statues.

In the 17th century the French physician Patin described the case of a local woman who had ‘turned to wood’. This ‘wood’ was actually bone and the woman possessed an incredibly rare condition that caused her muscles to be slowly turned to bone.

The earliest and most well documented case of fetal ossification dates back to 1582 when Mme Colombe Chatri died at the age of
sixty-eight, and a twenty-eight year old fetus was removed from her
womb. The "Stone-Child of Sens" should have been born in 1554; however,
labor came and went with no delivery and in the resulting decades the
fetus calcified and ossified
within the womb, which actually formed a shell. Mme Chatri seemed to
have lived a normal life, with the exception of regular abdominal pains. There will be more on the Stone Child in a subsequent post!

Fibrodysplasia Ossificans Progressiva or FOP, as the condition is know by today, affects 1 of 2 million people with varied severity. The condition is a genetic mutation in which the bodies of those affected cannot switch off the mechanism that grows the skeleton in the womb. Also, any small injury to connective tissue - muscles, ligaments, and tendons - results in the formation of hard bone around the damaged site. As it is a spontaneous genetic mutation, a FOB child can be born of normal parents – however persons with the condition have a 50% chance of passing the traits on to their offspring. As the hereotopic (extra) bone growth only becomes painfully obvious after a few years – 10 being the average - the only sign of the condition observable in an infant is malformed big toes. It is not until the second skeleton begins to form and mobility becomes severely restricted that the condition becomes evident.Persons with FOP have been involved in sideshow and curio displays for centuries. Those with the condition were commonly called ‘ossified men’ or ‘stone men’, ‘The Ossified Man’ became a popular attraction. An ossified woman named Miss Emma Shaler once even shared billing with Harry Houdini in 1894. Strangely enough the ossified individual became quite a common attraction – likely due to the fact that it was a Marvel easily faked.For those with the condition, life was far from easy. Movement was severely hampered and, in many cases, movement involved little more than lips and inner workings. The money these ossified men and women earned while on display paid for much needed medical attention. Many were often attended to during display by hired nurses. Few were able to eat anything, and their jaws became fused, and many had to sustain themselves on liquid diets. Thus many individual with the condition appeared incredibly gaunt and sickly. Most died quite young of pneumonia or other ailments that fed upon the sickly.Harry Raymond Eastlack (pictured above) was born in the early 1930’s and was one of the last modern ossified men presented as a curiosity. He died of pneumonia in 1973, and his case is particularly notable because shortly before his death, he made it known that he wanted to donate his body to science. The gesture was in the hopes that in death he would be able to help find a cure for this rare and somewhat cruel disease. As per his wishes, his preserved skeleton now resides in The Mütter Museum in Philadelphia. Mr. Eastlack remains the best documented Stone Man in history.Another FOP skeleton, belonging to a veteran Peter Cluckey, lives at the National Museum of Health and Medicine on the Walter Reed campus in Washington.From public affairs officer Steven Solomon and my friends at the Kircher Society:

Peter Cluckey was born in 1882, enlisted in the Army at age 17 just after the Spanish-American War, retired from the service after 3 years, and rejoined in 1904. Two months after his second enlistment he experienced joint pain and stiffness after a horseback mounted drill held in cold rain.

After several medical examinations he was diagnosed with “rheumatism chronic, articular, affecting both hips, knees, and ankle joints, and the right elbow.” Over the next 20 years his condition worsened to the point where every joint in his body became fused. Cluckey was moved into a sitting position so that he could be placed in a chair or on his side in bed to sleep. His front teeth were removed so that he could be fed soft foods. In his will, Cluckey donated his body to the museum and his skeleton has been on display seated in a wooden chair in the museum since his death in 1925.We invite you to visit this fascinating specimen in person. Admission and parking are free.Steven SolomonPublic Affairs OfficerNational Museum of Health and Medicine202-782-2200www.nmhm.washingtondc.museum